hrp0086rfc7.3 | Gonads & DSD | ESPE2016

Harmonisation of Serum Dihydrotestosterone Analysis: Establishment of an External Quality Assurance Program

Wudy Stefan , Hartmann Michaela , Jolly Lisa , Shun Ho Chung , Kam Richard , Joseph John , Boyder Conchita , Greaves Ronda

Background: Serum dihydrotestosterone (DHT) is an important analyte for the clinical assessment of disorders of sex development. It is also reportedly a difficult analyte to measure. Currently there are significant gaps in the standardisation of this analyte, including no external quality assurance (EQA) program available worldwide to allow for peer performance review of DHT.Objective and hypotheses: We therefore proposed to establish an EQA program for ...

hrp0086p1-p26 | Adrenal P1 | ESPE2016

Sex-Specific Differences in Hypothalamus-Pituitary-Adrenal Axis Activity in Newborns with Very Low Birth Weight

van der Voorn Bibian , de Waard Marita , Rotteveel Joost , Hartmann Michaela , van Goudoever Johannes , Lafeber Harrie , Wudy Stefan , Finken Martijn

Background: Male preterm infants are at increased risk of neonatal mortality when compared to their female counterparts. The mechanisms explaining this male disadvantage are not fully elucidated yet.Objective and hypotheses: To compare glucocorticoid metabolite excretion in urine obtained at day 10 between male and female infants born with a very low birth weight (VLBW; i.e. <1500 g). We hypothesized that male preterm infants have impaired adrenocort...

hrp0086p1-p343 | Gonads &amp; DSD P1 | ESPE2016

Evolution of Bone Mass and Body Composition in Gender Dysphoric Adolescents Treated with Progestins to Suppress Endogenous Hormones

Tack Lloyd , Craen Margarita , Lapauw Bruno , Goemaere Stefan , Toye Kaatje , Kaufman Jean-Marc , Vandewalle Sara , Zmierczak Hans-Georg , Cools Martine

Background: In gender dysphoric (GD) adolescents with advanced pubertal development, psychological relief can be attained with progestins, which are much cheaper and easier to administer than GnRHa. Moreover, use of GnRHa has been shown to interfere with pubertal bone mass accrual. To date, few data exist on the effects of progestins on body composition (BC) and bone parameters in this population.Objective and hypotheses: To explore the effects of pro- a...

hrp0086p2-p843 | Syndromes: Mechanisms and Management P2 | ESPE2016

The Specific Pubertal Height Gain is Higher in Boys as Well as in Children with Lower BMISDS

Holmgren Anton , Niklasson Aimon , Nierop Andreas , Gelander Lars , Aronson A. Stefan , Sjoberg Agneta , Lissner Lauren , Albertsson-Wikland Kerstin

Background: Growth in height during puberty can be described by the QEPS-model as a combination of continuous basal growth, QES, and a specific pubertal growth function, P.Objective and hypotheses: To study the relationship between childhood BMISDS and the prepubertal gain and pubertal gain related to growth functions from the QEPS-model.Method: The longitudinally followed GrowUpGothenburg 1990 birth co...

hrp0082p1-d2-156 | Growth (1) | ESPE2014

Gender Difference in Secular Trend in Sweden

Holmgren Anton , Niklasson Aimon , Nierop Andreas F M , Gelander Lars , Sjoberg Agneta , Aronson Stefan , Albertsson-Wikland Kerstin

Background, objective and hypotheses: By using QEPS, a new mathematic growth model, different components of growth can be analyzed, comparing secular trends of prepubertal and pubertal growth in Swedish birth cohorts born 1974 and 1990.Materials and methods: Two birth cohorts followed to adult height (AH) born around 1974 (1691 boys; 1666 girls) and 1990 (1647 boys; 1501 girls) being healthy, Nordic and born term. A subpopulation of 1974 (1177 boys; 1168...

hrp0084p2-473 | Growth | ESPE2015

The Pubertal Gain in Height is Inversely Related to BMI in Childhood

Holmgren Anton , Niklasson Aimon , Nierop Andreas F.M. , Gelander Lars , Aronson A. Stefan , Sjoberg Agneta , Lissner Lauren , Albertsson-Wikland Kerstin

Background: Weight in childhood may influence the pubertal timing and pattern of growth.Objective: To investigate the impact of BMI in childhood on further growth, especially the specific pubertal pattern of growth.Method: The longitudinally followed GrowUpGothenburg1990 birth cohort, was analyzed using the QEPS growth model (Nierop et al. Horm Res in Ped. 2013; 80(suppl 1):152–153) (describi...

hrp0094p2-429 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2021

4-year-old female patient with mixed germ cell tumor and underlying Cowden syndrome

Habacht Daniela , Boztug Heidrun , Engstler Gernot , Kager Leo , Boztug Kaan , Pauer Sophie , Amann Gabriele , Zeitlhofer Petra , Riedl Stefan

Cowden syndrome (CS) is a cancer predisposition syndrome. The clinical hallmarks of CS are macrocephaly and mucocutaneous lesions. Several tumor types have been described, mostly malignancies of the breast, thyroid, endometrium, kidney and colorectum. The age of onset is extremely variable. So far, only two case reports of children with ovarian tumors in the context of CS have been published, a granulosa cell tumor of a 16-year-old girl (Smpokou et al. 2014) and a bilateral en...

hrp0097p1-496 | GH and IGFs | ESPE2023

Comparative Efficacy of Growth Hormone treatment on children with Idiopathic Growth Hormone Deficiency (and Idiopathic Short Stature (A 12year Tertiary Center Experience

Ahmed Fatima , Hammouri Marwa , Allami Zahraa , Al Jneibi Sara , Al Jubeh Jamal , Weber Stefan , Al Remeithi Sareea

Short stature is one of the most common concerns presented to pediatric endocrinologists. ISS describes a heterogeneous group of children with a height of more than 2 SD score (SDS) below the corresponding mean height for a given age, sex, and population group without underlying aetiology. The primary objectives of GH treatment are acceleration of growth velocity to promote normalization of stature during childhood and attainment of normal FAH.Ob...

hrp0084lbp-1256 | Late Breaking Posters | ESPE2015

Higher Expression of the Oncogene YAP1, a Wnt/β-Catenin Target, is associated with Poor Outcome in Pediatric Patients with Adrenocortical Tumors

Abduch Rafael H , Bueno Ana Carolina , Leal Leticia F , Cavalcanti Marcelo M , Brandalise Silvia R , Masterallo Maria J , Yunes Jose A , Martinelli Jr Carlos E , Scrideli Carlos A , Tone Luiz G , Tucci Silvio , Moreira Ayrton C , Ramalho Leandra Z , Castro Margaret , Antonini Sonir R

Background: overexpression of the oncogene Yes-associated protein 1 (YAP1), a Hippo pathway target, associates with increased cell proliferation in some human cancers. There is not data on adrenocortical tumors (ACT). YAP1 is a potential target of Wnt/β-catenin pathway, which plays an important role in ACTs.Objective and hypotheses: to evaluate the role of YAP1 and its interaction with the Wnt/β-catenin pathway in ACT.Met...

hrp0095fc5.5 | Adrenals and HPA Axis | ESPE2022

Influence of prenatal environment and genetic background on glucocorticoid steroid metabolism in monozygotic twins with intra-twin birthweight-differences

Schulte Sandra , Schreiner Felix , Plamper Michaela , Kasner Charlotte , Gruenewald Mathias , Bartmann Bartmann Peter , Fimmers Rolf , Hartmann Michaela F. , Wudy Stefan A. , Woelfle Joachim , Gohlke Bettina

Preliminary results:Objective: Although low birthweight (bw) and unfavourable intrauterine conditions have been associated with metabolic sequelae in later life, little is known about their impact on steroid metabolism. We studied genetically identical twins with intra-twin bw-differences from birth to adolescence to analyse the long-term impact of bw on steroid metabolism.Methods:...